Guillain-Barre Syndrome

1. Guillain-Barre Syndrome Presentor: Izaskun C. Ganao Moderator: Dr. Mary Katherine E. Millan-Morales Reactor: Dr. Teresita N. Rabanal

2. Objectives • To discuss the approach to a patient with Guillain-Barre Syndrome • To discuss the clinical presentation and management of Guillain-Barre Syndrome

3. IDENTIFYING DATA • JSS • 4 year old • Male • Filipino • From Mandaluyong • Admitted for the 1sttime in our institution last September 30 2013

4. Chief Complaint Vomiting And Doubling of Vision

5. History of Present Illness 1 day PTA Patient complained of doubling of vision noted to have inward deviation of the eyes, L>R (-) vomiting (-) history of trauma (-) headache (-) dizziness (-) body weakness (-) seizure Spontaneous resolution of symptoms

6. History of Present Illness Morning PTA doubling of vision associated with inward deviation of the right eye (+) 4 episodes of vomiting (-) body weakness (-) headache (-) numbness Consult at TMC-ER Advised admission

7. Review of Systems • Skin(-) jaundice, (-) active dermatoses, (-) pruritus, photosensitivity • Eyes(-) lacrimation, (-) preferential gaze, (-) strabismus, (-) blurring of vision, lacrimation, redness • Ears:(-) discharge, (-) tenderness, (-) ear pain, deafness, tinnitus • Nose: (-) epistaxis, (-) discharge • Mouth: (-) sores, fissures, bleeding gums • Neck: (-) stiffness, limitation of motion on the neck • Pulmonary: (-) hemoptysis, (-) difficulty of breathing • Cardiac: (-) chest pain, orthopnea, syncope • Gastrointestinal: (-) constipation (-) hematochezia , (-) melena • Genitourinary: (-) urgency, hesitancy, dysuria, hematuria, frequency • Muscular: (-) swelling, (-) trauma, (-) wasting • Hematopoietic: (-) bleeding, (-) easy bruising • Endocrine: (-) polydipsia, polyuria, polyphagia, nocturia, (-) heat intolerance tremors • Neuro: (-) headache, seizure, head trauma

8. Past Medical History • (+) Skin Asthma • (+) Measles – 2011 (2 years old) • No previous head trauma • No known allergies • No previous surgeries • Newborn screen – normal

9. Family History • (+) Asthma • (+) Diabetes

10. Birth History • Full term • Normal spontaneous delivery • 31 year old G1P1 (1001) • Birth weight: 2.8 kg • No feto-maternal complications

11. Nutritional History • No particular food preferences • No known food allergies

12. Immunization History • BCG - 1 dose • DPT/OPV - 3 doses + 2 boosters • HiB- 3 doses • Hepatitis-B - 3 doses • MMR - 2 doses • Varicella - 1 dose • PCV - 3 doses • Hepatitis - 2 doses • Typhoid - 1 dose • Influenza – Last dose: 2012

13. Physical Examination at the ER • General survey – Awake, active, not in cardio-respiratory distress • Vital signs: BP: 90/60 CR: 82 bpm RR: 26 cpm T: 36.1⁰C Weight: 16 kg Height: 105 BMI: 14.5

14. Physical Examination at the ER • Nonsunken eyeballs, moist lips, no oral sores • Symmetrical chest expansion, no retractions, clear breath sounds • Adynamic precordium, normal rate, regular cardiac rhythm, no murmurs • Flat abdomen, normoactive bowel sounds, soft abdomen, nontender • Full pulses, CRT less than 2 seconds, warm extremities

15. Physical Examination at the ER • Neurologic Examination • GCS 15, awake, follows commands, coherent, able to speak and comprehend spoken language

16. Cranial nerves: • CN I – intact • CN II, III – pupils equally briskly reactive to light • CN III – no ptosis, pupils equally briskly reactive to light • CN IV – no diplopia on looking down or in • CN VI – diplopia on lateral gaze to the right • CN V – intact corneal reflex • CN VII – no facial asymmetry • CN VIII – intact gross hearing • CN IX, X - can swallow, intact gag • CN XI – good shoulder shrug • CN XII – tongue midline

17. Physical Examination at the ER • Neurologic Examination • CN V – intact corneal reflex • CN VII – no facial asymmetry • CN VIII – intact gross hearing • CN IX, X - can swallow, intact gag • CN XI – good shoulder shrug • CN XII – tongue midline

18. Physical Examination at the ER • Neurologic Examination Motor: Sensory: Deep tendon reflexes:

19. (-) Babinski • (-) Clonus • Supple neck, non meningeal signs

20. Admitting Impression Vomiting, unspecified; t/c abduction deficit, OD more than OS, probably secondary to cranial nerve VI compression ; R/O Intracranial mass

21. Laboratory Examinations

22. Therapeutics: • Eye patch application for 4 hours each eye alternately • Vitamin B complex once a day

23. 1st hospital day

24. Laboratory Examinations • HGT: 102 mg/dL • ECG: sinus bradycardia, normal sinus rhythm • Chest x-ray: no significant chest findings • MRI of the orbits: polysinusitis, enlarged adenoids, unremarkable MRI of the orbits

25. Second Hospital Day

26. Third Hospital Day

27. Laboratory Examinations • EMG-NCV of the lower extremities: normal findings for age except for the bilateral absence of the H-reflex. Consider beginning or early polyneuropathy.

28. Third Hospital Day, PICU

29. Laboratory Examinations • Electromyography/Nerve conduction Study of the lower extremities • normal findings for age except for the bilateral absence of the H-reflex • Electrical equivalent of the tendon jerk • ECG • sinus arrhythmia

30. Fourth Hospital Day, PICU

31. Fifth Hospital Day, PICU

32. Sixth Hospital Day, PICU

33. Seventh Hospital Day, PICU

34. Eighth Hospital Day, PICU

36. Tenth Hospital Day

37. Eleventh Hospital Day

38. Thirteenth Hospital Day

39. Sixteenth Hospital Day

40. Final Diagnosis Guillain-Barre Syndrome, Miller-Fisher Variant; s/p IVIg; Polysinusitis

41. discussion

42. Salient Features • 4 year old male • Afebrile • Doubling of vision • Vomiting • Abdominal pain • Constipation • Ascending weakness of the extremities • Areflexia of the lower extremities • Limitation of movement of extraocular muscles • Arrhtyhmia and bradycardia • Ptosis • Headache • Difficulty swallowing liquids • Hoarseness of the voice

43. Differential DiagnosEs

44. Intracranial mass • headache, • altered mental status • ataxia • nausea, vomiting • weakness, and gait disturbance • focal seizures • fixed visual changes • speech deficits • focal sensory abnormalities

45. Botulism • Nausea • Vomiting • Extremely dry mouth unrelieved by drinking fluids • Sore throat • Cranial nerve paralysis manifesting as: • blurred vision • diplopia • Ptosis • extraocularmuscle weakness or paresis • fixed/dilated pupils • dysarthria • dysphagia or suppressed gag reflex

46. Botulism • Paralytic ileus advancing to severe constipation • Gastricdilatation • Bladder distention advancing to urinary retention • Orthostatic hypotension • Reduced salivation • Reduced lacrimation

47. Botulism • Changes in deep tendon reflexes, which may be either intact or diminished • Incoordination due to muscle weakness • Absence of pathologic reflexes and normal findings on sensory and gait examinations • Normal results on mental status examination

48. Botulism • Symmetrical descending paralysis or weakness of motor and autonomic nerves • Respiratory muscle weakness subtle or progressive, advancing rapidly to respiratory failure

49. Poliomyelitis • Fever • Headache • Nausea/Vomiting • Abdominal pain • Oropharyngealhyperemia • Nuchal rigidity • Back and lower extremity pain • Meningitis with lymphocytic pleocytosis (usually) Paralytic poliomyelitis occurs in fewer than 5% of affected patients and is characterized by the following: • Compromise of the motor neurons may be localized or widespread. • asymmetric loss of muscle • Muscle atrophy is generally observed several weeks after the beginning of symptoms.

50. Myasthenia Gravis • specific muscle weakness rather than generalized muscle weakness • The severity of the weakness typically fluctuates over hours being least severe in the morning and worse as the day progresses • increased by exertion and alleviated by rest • The degree of weakness also varies over the course of weeks or months, with exacerbations and remissions • Extraocular muscle weakness or ptosis is present initially in 50% • Patients progress from mild to more severe disease over weeks to months. • Weakness tends to spread from the ocular to facial to bulbar muscles and then to truncal and limb muscles